Table 1 Clinical profiles of 23 patients without corticosteroid treatment

Patient no.	Age at onset	Sex	Obstructive jaundice	Abdominal pain	Pancreatic ductal change	Total bilirubin (mg/dl)	Alkaline phosphatase (IU/l)	IgG4 (mg/dl)	BT–PABA (%)	Initial treatment	Event during follow-up	Period to event occurence or observation period (months)
1	70	M	+	+	Diffuse	4.1	800			EBD	OJ	5
2	66	M	+	–	Diffuse	3.6	605			EBD	SC	40
3	69	M	+	–	Diffuse	7.4	848	305	72	EBD	SC	4
4	49	M	+	–	Diffuse	6.9	640	222	52	EBD	SC	12
5	80	M	+	–	Diffuse	1.6	1975			EBD	SC	4
6	56	M	–	–	Local	0.7	174			None	OJ	19
7	79	M	+	+	Diffuse	3.3	1949	420	61	EBD	SC	1
8	70	M	+	–	Diffuse	5.8	1126	2170		EBD	OJ	5
9	41	M	–	+	Local	0.5	197			DP	OJ	18
10	76	M	+	–	Diffuse	14.1	778	65.2		EBD	SC	1
11	65	F	–	–	Diffuse	0.6	219	374	70	None	Appearance of pseudocyst	30
12	68	M	+	+	Local	2.0	1546		27	None	SC	24
13	70	M	+	–	Diffuse	4.1	581			PTBD	SC	89
14	65	M	+	–	Diffuse	2.1	1632			None	SC	92
15	64	M	–	+	Local	0.5	877	670	70	None	Interstitial nephritis	13
16	66	M	–	–	Local	0.4	202	135		DP	Retroperitoneal fibrosis	43
17	76	M	–	–	Local	0.8	339	812		None	None	79
18	75	M	–	–	Diffuse	0.7	164	770		None	None	45
19	79	M	–	–	Local	0.6	168	554	69	None	None	43
20	68	F	–	–	Local	0.6	192	553	72	None	None	59
21	58	F	–	–	Local	0.6	139	592	69	None	None	36
22	61	F	–	–	Diffuse	0.4	183	354	80	None	None	22
23	52	M	–	–	Local	0.7	112	164		PD	None	10

BT–PABA, N-benzoyl-l-tyrosyl-p-aminobenzoic acid test >70% normal; DP, distal pancreatectomy; EBD, endoscopic biliary drainage; OJ, obstructive jaundice caused by intrapancreatic bile duct stenosis; PD, pancreatoduodenectomy; PTBD, percutaneous transhepatic biliary drainage; SC, sclerosing cholangitis. Normal range: Total bilirubin, 0.3–1.3 mg/dl; alkaline phosphatase, 60–201 IU/l; IgG4, <135 mg/dl.